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Cited by 336

The DOI matches its Crossref record; 77 of the 77 references it cites were found in a registry or an index.

Fibrosing interstitial lung diseases: knowns and unknowns

Open access
Cottin, Vincent, Wollin, Lutz, Fischer, Aryeh, Quaresma, Manuel, Stowasser, Susanne, Harari, Sergio·2019·European Respiratory Review

Abstract

Patients with certain types of fibrosing interstitial lung disease (ILD) are at risk of developing a progressive phenotype characterised by self-sustaining fibrosis, decline in lung function, worsening quality of life, and early mortality. It has been proposed that such progressive fibrosing ILDs, which show commonalities in clinical behaviour and in the pathogenetic mechanisms that drive progressive fibrosis, may be “lumped” together for the purposes of clinical research and, potentially, for treatment. At present, no drugs are approved for the treatment of ILDs other than nintedanib and pirfenidone for the treatment of idiopathic pulmonary fibrosis. For other progressive fibrosing ILDs, the mainstay of drug therapy is immunosuppression. However, it is postulated that, once the response to lung injury in fibrosing ILDs has reached the stage at which fibrosis has become progressive and self-sustaining, targeted antifibrotic therapy would be required to slow disease progression. Nintedanib, an intracellular inhibitor of tyrosine kinases, has shown antifibrotic, anti-inflammatory and vascular remodelling effects in several non-clinical models of fibrosis, irrespective of the trigger for the injury. Ongoing clinical trials will provide insight into the role of antifibrotic treatment with nintedanib or pirfenidone in the management of fibrosing ILDs with a progressive phenotype.

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Metadata

Authors
Cottin, V., Wollin, L., Fischer, A., Quaresma, M., Stowasser, S., & Harari, S.
Venue
European Respiratory Review
Published
2019-02-27
Volume
28
Issue
151
Pages
180100
ISSN
  • 0905-9180 (ISSN-L)
  • 1600-0617
OpenAlex
W2920766120
Cited by
336
References
77
FWCI
24.38
Type
journal article
Language
en
Open access
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CoChat. (2026). Citation check: Fibrosing interstitial lung diseases: knowns and unknowns. https://app.cochat.ai/papers/10.1183/16000617.0100-2018
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Cottin, V., Wollin, L., Fischer, A., Quaresma, M., Stowasser, S., & Harari, S. (2019). Fibrosing interstitial lung diseases: knowns and unknowns. European Respiratory Review. https://doi.org/10.1183/16000617.0100-2018

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